|Year : 2020 | Volume
| Issue : 3 | Page : 148-151
Colonic Burkitt's lymphoma related to bowel obstruction in adults: A case report and literature review
Shayma Misfer Alotaibi1, Abdulrahman Misfer Alotaibi1, Salma Hani Sait1, Najla Mohammedsaleh Alghanmi2, Abdulrahman Mohammed Sibiany3
1 Department of General Surgery, King Abdulaziz University Hospital, Jeddah, Saudi Arabia
2 Department of Pathology, King Abdulaziz University Hospital, Jeddah, Saudi Arabia
3 Department of General Surgery, Faculty of Medicine, King Abdulaziz University Hospital, King Abdulaziz University, Jeddah, Saudi Arabia
|Date of Submission||07-Dec-2019|
|Date of Acceptance||22-Apr-2021|
|Date of Web Publication||19-Jul-2021|
Dr. Shayma Misfer Alotaibi
King Abdulaziz University, Jeddah
Source of Support: None, Conflict of Interest: None
Burkitt's lymphoma (BL) is an uncommon cause of non-Hodgkin lymphoma in adults. BL-related intussusception causing intestinal obstruction in adults is rare. Symptoms are often misleading and make diagnosis difficult. We aim to present an adult case of a Colonic BL related to bowel obstruction. Here, we are presenting a case of a 29-year-old Saudi male patient who presented with symptoms of bowel obstruction. Abdominal examination found abdominal distention with a palpable mass in the right lower quadrant with mild tenderness. After chest, abdomen, and pelvic enhanced computed tomography with intravenous contrast, colonoscopy, and histopathological examination, the patient was diagnosed with colonic BL, which caused him bowel obstruction. Chemotherapy has been started based on Hyper CVAD chemotherapy protocol. After receiving the complete Chemotherapy on July 2019, patient's symptoms improved; there is no longer abdominal pain; he is passing stool, usually with no blood or mucus. Two months post last cycle of chemotherapy the patient presented again to the hospital with symptoms and images showed refractory disease to first-line chemotherapy. He passed away one month after this admission.
Keywords: Bowel, Burkett's lymphoma, non-Hodgkin lymphoma, obstruction
|How to cite this article:|
Alotaibi SM, Alotaibi AM, Sait SH, Alghanmi NM, Sibiany AM. Colonic Burkitt's lymphoma related to bowel obstruction in adults: A case report and literature review. Saudi Surg J 2020;8:148-51
|How to cite this URL:|
Alotaibi SM, Alotaibi AM, Sait SH, Alghanmi NM, Sibiany AM. Colonic Burkitt's lymphoma related to bowel obstruction in adults: A case report and literature review. Saudi Surg J [serial online] 2020 [cited 2023 Feb 6];8:148-51. Available from: https://www.saudisurgj.org/text.asp?2020/8/3/148/321725
| Introduction|| |
Burkitt lymphoma (BL) is an aggressive subtype of non-Hodgkin's lymphoma. It is recognized as the fastest growing human tumor, as it is associated with impaired immunity and is rapidly fatal if left untreated. It accounts for approximately 0.3%–1.3% of all non-Hodgkin lymphomas. Three clinical forms of BL are described: endemic, sporadic, and immunodeficiency-associated. Endemic BL refers to those cases occurring in Africa, usually in children who present with the involvement of multiple nodal and extranodal sites. Sporadic BL has no geographic predilection and occurs worldwide; it accounts for 1% to 2% of lymphomas in the adult population. Immunodeficiency-associated BL occurs mainly in patients infected with HIV, transplant recipients are taking immunosuppressive, and in people with congenital immune-deficiencies. Very few cases of rectal BL have been reported in adults.,,, We aimed to present a case and review the literature of colonic BL related to bowel obstruction.
| Case Report|| |
A 29-year-old Saudi male smoker patient, not known to have any medical or surgical history, presented with severe abdominal pain for three weeks. The pain was generalized and intermittent, associated with abdominal distention, nausea, and vomiting, constipation, passing only flatus for a month, and a minimal amount of stool mixed with mucus and blood two days before presentation, he received over the counter analgesics, antacids, and laxatives without improvement. The patient had a positive history of weight loss; he lost 12 kg in 24 days. There was no fever or jaundice, and other systematic review was unremarkable. He does not have family history of inflammatory bowel disease, lymphoma, or colon cancer.
On physical examination, there was abdominal distention with a palpable mass in the right lower quadrant with mild tenderness; the per-rectal examination was unremarkable with stool without blood or mucus. The laboratory investigations did not show significant disorders where the hemoglobin concentration was 11.3 g/dL, the white blood cell count was 8.3 × 109/L, and the carcinoembryonic antigen was 0.5 ng/mL.
The enhanced computed tomography (CT) abdomen and pelvis with intravenous (IV) contrast scan reported right ileocecal soft-tissue mass, multiple enlarged abdominopelvic lymph nodes, amalgamated lymph nodes in the right mid-abdomen, and omental involvement. It finally concluded that the disease involves the bowel, lymph nodes, and peritoneal reflections (peritoneal lymphomatosis) [Figure 1].
|Figure 1: Enhanced CT abdomen and pelvis with IV contrast showed right ileocecal soft tissue mass, multiple enlarged abdominopelvic lymph nodes, amalgamated lymph nodes in the right mid-abdomen and omental involvement|
Click here to view
The enhanced CT chest with IV contrast scan showed mediastinal, right hilar, bilateral internal mammary, and cardiophrenic metastatic lymphadenopathy and signs of bilateral pleural effusion with nodular pleural thickening likely due to pleural metastasis [Figure 2].
|Figure 2: Enhanced CT chest with IV contrast showed mediastinal, right hilar, bilateral internal mammary, and cardiophrenic metastatic lymphadenopathy|
Click here to view
Colonoscopy was done, and showed fresh blood in the entire examined colon, (5 cm × 5 cm) malignant appearing mass at the cecum, distorted anatomy at the cecum [Figure 3].
Histopathology showed tumor cells are CD20 positive with almost 100% proliferation index by ki67, high-grade B-cell lymphoma consistent with BL [Figure 4].
Chemotherapy treatment has been started based on Hyper CVAD chemotherapy protocol. Total of eight chemotherapy cycles have been received (Four cycles of Arms A and four cycles of arm B). His first cycle was on January 9, 2019. The last one was on July 10, 2019. Post chemotherapy he had febrile neutropenia requiring IV antibiotics and supportive transfusions.
During his multiple admissions, CT chest, abdomen, and pelvis have been done to see the progression of the disease. Last image was done on April 4, 2019 showed; significant interval improvement of the overall illness with residual right paracolic lymph nodes, which are smaller in size lower which may suggest interval necrosis, interval improvement of the previously noted peritoneal disease and ascites and significant regression of the size and number of the mediastinal and internal mammary lymph nodes in comparison to the substantial progression of the pulmonary nodules.
Clinically, patient's symptoms improved; there is no longer abdominal pain; He is passing stool, usually with no blood or mucus. Patients' appetite had improved as well as his nutritional status, and he had gained weight.
Until two months post last cycle of chemotherapy on November 23, 2019 the patient presented again to the hospital with symptoms and images showed refractory disease to first-line chemotherapy. He passed away one month after this admission.
| Discussion|| |
Gastrointestinal lymphoma is considered 5%–10% of all non-Hodgkin's lymphoma, with intestinal lymphoma contributing 15%–20% of all gastrointestinal lymphoma. Most of these arise in the stomach (65%), followed by the small bowel (20%–30%) with rest arising in the rectum and colon., B-cell lymphoma of the colon is the third known malignancy of the colon after carcinoma and carcinoid though its prevalence is <0.5%.
The involvement of caecum within the colon is the most popular, and Gonzalez et al. found caecum (60%) to be the most frequent site, followed by the right (27%) and the sigmoid colon (13%). Bairey et al., in their case series of 17 patients, also found the ileocaecal region to be the common site of colonic lymphoma, accounting for 76% of cases. In our case, the enhanced CT abdomen and pelvis with IV contrast scan reported right ileocecal soft tissue mass.
B-cell lymphomas are categorized into diffuse large b-cell lymphoma, extranodal marginal zone lymphoma (mucosa-associated lymphoid tissue-associated lymphoma), mantle cell lymphoma, BL, and follicular lymphoma. Our case was diagnosed as high-grade B-cell lymphoma consistent with BL.
Primary colorectal lymphoma mainly affects the older age group in the fifth to the seventh decades of life with a male:female ratio being 1.5:1. Wong and Eu, in their study of 14 cases, found the mean age of presentation to be 61 years, while Bairey et al. found it to be 72 years. In contrast to the above studies, our patient presented with lymphoma at 29 years of age.
The presentation of B cell lymphoma is varied, and symptoms relayed on the site of the lesion. Most commonly, abdominal pain, abdominal mass, weight loss, and hematochezia, besides the features of obstruction like nausea, vomiting, change in bowel habits,, obstruction,, intussusceptions, and acute peritonitis due to intestinal perforation,, are present. Fan et al., in their series of 37 patients, reported weight loss and abdominal pain in 43% and 62% of cases, respectively. In contrast, Bairey et al. reported weight loss and abdominal pain\respectively in 29% and 56% of cases. Intestinal obstruction is an infrequent presentation due to the more pliable nature of colorectal lymphoma and the absence of desmoplastic response; despite this, symptoms of partial obstruction are more frequent. These matched with our case, where he was presented with severe abdominal pain, nausea and vomiting, constipation, and weight loss.
We followed the National Comprehensive Cancer Network guidelines for the adaptation of colonic lymphoma.
Colonoscopy is helpful in the diagnosis of primary lymphoma of the colon, but it is not always possible to determine this type of tumor due to an inadequate biopsy. More than half of the patients present with the bulky disease usually >5 cm. The histopathological examination of tumor cells in BL generally strongly express markers of B cell differentiation (CD20, CD22, CD19), as well as CD10 and BCL6. The tumor cells are usually negative for BCL2 and TdT. The high mitotic activity of BL is confirmed by nearly 100% of the cells staining positive for Ki67.
Because of the low incidence of the disease, the optimal management of primary lymphoma of the colon and rectum has never been determined by randomized trials. A study by Tondini et al. suggested that the principal modality of treatment is combined modality treatment that includes surgery and chemotherapy. Early-stage tumors are treated with surgery, followed by polychemotherapy. The combination of rituximab to standard CHOP regimen has led to the development of progression-free, event-free, disease-free, and overall survival., Surgery is the first mode of therapy for palliation of pain and new conditions like obstruction, perforation, and bleeding, as reported by Cai et al. and Zhai et al.,
| Conclusion|| |
Colonic lymphoma is a rare clinical entity presenting most commonly in an older age group. The presentation is often nonspecific, which leads to delayed diagnosis and advanced stage at the exhibition. Surgery is the mainstay of treatment for early disease, possibly followed by chemotherapy and radiation. Complicated disease requires surgery includes; obstruction, perforation, and bleeding. Advanced disease may benefit from chemotherapy.
Written informed consent was obtained from the patient for publication of this case report. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understand that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Otieno MW. Non-endemic burkitt's lymphoma. In: Burkitt's Lymphoma. New York, NY: Springer; 2013. p. 121-30.
Blum KA, Lozanski G, Byrd JC. Adult Burkitt leukemia and lymphoma. Blood 2004;104:3009-20.
Greif F, Burstein Y, Hammer B. Burkitt's lymphoma protruding through the anus. Report of an unusual case. Dis Colon Rectum 1988;31:629-31.
Matković S, Jelić S, Manojlović N, Milanović N. Non-Hodgkin's lymphomas with primary localization in large bowel and rectum. Med Sci Monit 2000;6:68-74.
Palakodeti S, Hoda K, Munroe CA. Perirectal Burkitt lymphoma presenting as an uncommon cause of lower gastrointestinal bleeding. Clin Gastroenterol Hepatol 2014;12:A23-4.
Dinh MH, Matkowskyj KA, Stosor V. Colorectal lymphoma in the setting of HIV: Case report and review of the literature. AIDS Patient Care STDS 2009;23:227-30.
Zinzani PL, Magagnoli M, Pagliani G, Bendandi M, Gherlinzoni F, Merla E, et al
. Primary intestinal lymphoma: Clinical and therapeutic features of 32 patients. Haematologica 1997;82:305-8.
Bairey O, Ruchlemer R, Shpilberg O. Non-Hodgkin's lymphomas of the colon. IMAJ Ramat Gan 2006;8:832.
Gonzalez QH, Heslin MJ, Dávila-Cervantes A, Alvarez-Tostado J, de los Monteros AE, Shore G, et al
. Primary colonic lymphoma. Am Surg 2008;74:214-6.
Harris NL, Jaffe ES, Diebold J, Flandrin G, Muller-Hermelink HK, Vardiman J. Lymphoma classification – From controversy to consensus: The R.E.A.L. and WHO Classification of lymphoid neoplasms. Ann Oncol 2000;11 Suppl 1:3-10.
Ismaili N, Bensouda Y, Mellas N, Errihani H. Role of chemotherapy in the management of primary rectal lymphoma: A case report and review of the literature. Cases J 2009;2:9373.
Wong MT, Eu KW. Primary colorectal lymphomas. Colorectal Dis 2006;8:586-91.
Chiang JM, Lin YS. Tumor spectrum of adult intussusception. J Surg Oncol 2008;98:444-7.
Fan CW, Changchien CR, Wang JY, Chen JS, Hsu KC, Tang R, et al
. Primary colorectal lymphoma. Dis Colon Rectum 2000;43:1277-82.
Zelenetz AD, Gordon LI, Wierda WG. NCCN clinical practice guidelines in oncology. B Cell Lymphomas 2017.
Quayle FJ, Lowney JK. Colorectal lymphoma. Clin Colon Rectal Surg 2006;19:49-53.
Norris D, Stone J. WHO Classification of Tumors of Hematopoietic and Lymphoid Tissues. Geneva: WHO; 2008. p. 22-3.
Tondini C, Giardini R, Bozzetti F, Valagussa P, Santoro A, Bertulli R, et al
. Combined modality treatment for primary gastrointestinal non-Hodgkin's lymphoma: The Milan Cancer Institute experience. Ann Oncol 1993;4:831-7.
Morrison VA. Evolution of R-CHOP therapy for older patients with diffuse large B-cell lymphoma. Expert Rev Anticancer Ther 2008;8:1651-8.
Cai S, Cannizzo F Jr., Bullard Dunn KM, Gibbs JF, Czuczman M, Rajput A. The role of surgical intervention in non-Hodgkin's lymphoma of the colon and rectum. Am J Surg 2007;193:409-12.
Zhai L, Zhao Y, Lin L, Tian Y, Chen X, Huang H, et al.
Non-Hodgkin's lymphoma involving the ileocecal region: A single-institution analysis of 46 cases in a Chinese population. J Clin Gastroenterol 2012;46:50914.
[Figure 1], [Figure 2], [Figure 3], [Figure 4]